General anesthesia of a Japanese infant with Barber-Say syndrome: a case report.
General anesthesia of a Japanese infant with Barber-Say syndrome: a case report.
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DOI：
10.1186/s40981-016-0033-x
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发表时间：
2016
影响因子：
0.9
通讯作者：
Tanaka K
中科院分区：
文献类型：
--
作者：
Hamaguchi E;Tsutsumi YM;Kume K;Sakai Y;Kakuta N;Uemura Y;Kawahito S;Tanaka K
Barber-Say综合征（BSS）是一种非常罕见的先天性疾病，其特征是巨口，皮肤拉克萨和其他特征。我们报告我们的经验进行全身麻醉的日本儿童BSS。计划在全身麻醉下为一名18个月大的巨口症男性进行双侧嘴角修复术;该儿童身高75 cm，体重9.9 kg。由于外周静脉导管插入困难，由儿科医生在手术前插入。患者佩戴口罩，在静脉麻醉失去意识后手动通气。成人用口罩具有上级贴合性，可有效防止嘴角漏气。罗库溴铵给药后，用Macintosh喉镜扩张喉部。没有喉部解剖异常，气管插管是容易的。手术顺利完成。手术结束后一小时，双臂僵硬数秒，但患者未出现其他并发症。在BSS患者的全身麻醉中，面罩通气和静脉导管插入可能很困难，麻醉管理需要谨慎。
Barber-Say syndrome (BSS) is a very rare congenital disorder characterized by macrostomia, cutis laxa, and other features. We report our experience of performing general anesthesia on a Japanese child with BSS. A bilateral repair of the corners of the mouth under general anesthesia was planned for an 18-month-old male with macrostomia; the child was 75 cm in height and weighed 9.9 kg. As insertion of the peripheral intravenous catheter was difficult, it was inserted before the surgery by a pediatrician. The patient wore a mask and was ventilated manually after loss of consciousness with intravenous anesthesia. A mask for adults provided a superior fit and was effective in preventing air leakage from the corners of the mouth. After rocuronium was administered, the larynx was spread with a Macintosh laryngoscope. There was no laryngeal anatomical abnormality, and tracheal intubation was readily possible. The operation was completed without incident. Stiffening of both arms occurred for several seconds one hour after the operation ended, but the patient did not develop other complications. Mask ventilation and the insertion of an intravenous catheter may be difficult in the general anesthesia of patients with BSS, and anesthetic management requires caution.